Complete tracheal rings removed from a child’s airway during tracheal resection.Complete tracheal rings are a congenital defect in the cartilage rings which result in a narrowing of the tracheal opening. A normal tracheal cartilage is C-shaped with a softer, posterior membrane consisting of muscle. In complete tracheal rings, the trachea consists of several or more complete rings of cartilage with a narrowed diameter.
This is thought by some to occur from abnormal growth of the tracheal cartilage as compared to the muscular part of the normal trachea. The affected airway may range from involvement of just a few rings to most of the trachea and can extend into the left or right lung through the bronchi.
Endoscopic view of complete tracheal rings.Children with complete tracheal rings may have associated malformations, cardiac and pulmonary anomalies, Down’s syndrome, and Pfeiffer syndrome. It can be associated with a vascular anomaly known as a “ring-sling” complex in which the pulmonary artery due to an abnormal course can sling around the trachea and cause compression and narrowing of the tracheal opening.
Complete tracheal rings usually presents in the neonatal or infant period. It is diagnosed less often later in childhood, with symptoms developing at a few months of age. Some children may present in acute respiratory distress. Affected children may experience rapidly worsening symptoms in the face of an upper respiratory infection. Symptoms include:
Evaluation may include X-rays and more advanced imaging such as CT or MR imaging. Imaging can provide information not only about the extent of airway narrowing, but also any other lesions that might be present such as cardiac or vascular problems.
Microlaryngoscopy and bronchoscopy is essential to fully diagnose and characterize complete tracheal rings. This diagnostic test provides clarification of the tracheal lesion and differentiates it from lesions in the larynx that can cause stridor and respiratory distress. Furthermore, the test can help show the degree of narrowing and length of narrowing in the trachea.
In addition to airway evaluation, patients may also require evaluations by pulmonologists and gastroenterologists. Illnesses in the lungs or esophagus/stomach can affect the overall health of the patient and surgical outcomes. Advanced evaluation may include endoscopic examinations by these specialty services at the same time as a microlaryngoscopy and bronchoscopy.
Treatment of complete tracheal rings depends on the extent of the narrowing, caliber of the airway, and condition of the child.
Milder forms of complete tracheal rings can sometimes be monitored with close observation at regular doctor’s visits. Larger caliber rings in children with less severe symptoms may grow and require no surgical intervention.